Usual Adult Dose for:

Usual Pediatric Dose for:

Additional dosage information:

Usual Adult Dose for Wolman's Disease

1 mg/kg IV every other week

Use: For the treatment of lysosomal acid lipase (LAL) deficiency

Usual Pediatric Dose for Wolman's Disease

1 month or older:
1 mg/kg IV every other week

Rapidly progressive LAL deficiency presenting within first 6 months of life:
-Initial dose: 1 mg/kg IV once a week
-Maintenance dose: May increase to 3 mg/kg IV once a week if optimal clinical response is not achieved

Use: For the treatment of LAL deficiency

Renal Dose Adjustments

Data not available

Liver Dose Adjustments

Data not available

Precautions

Safety and efficacy have not been established in patients younger than 1 month.

Consult WARNINGS section for additional precautions.

Dialysis

Data not available

Other Comments

Administration advice:
-For IV infusion only
-Administer the diluted solution using a low protein binding infusion set with an in-line, low protein binding 0.2 micron filter.
-Infuse over at least 2 hours; consider a longer infusion time in patients receiving the 3 mg/kg dose or those who have had hypersensitivity reactions.
-May consider an infusion rate of 1 hour in patients receiving the 1 mg/kg dose who tolerate the infusion
-Consult the manufacturer product information for recommended infusion volumes.

Storage requirements:
-Do not freeze or shake.
-Protect from light.
-Use diluted solution immediately; if cannot use immediately, may store for up to 24 hours at 2C to 8C (36F to 46F)